Effectiveness of intravenous infliximab versus subcutaneous adalimumab in Takayasu arteritis: multicenter retrospective study.

Objectives: In this large multicentre study, we aimed to compare the effectiveness of intravenous infliximab vs subcutaneous adalimumab in patients with Takayasu arteritis.

Methods: We conducted a retrospective multicentre study across referral centers in France, Italy, Spain, Armenia, Israel, Japan, Tunisia, and Russia, analyzing biological-targeted therapies in TAK from January 2017 to September 2019.

Results: A total of 135 TAK patients who received adalimumab (n = 34) or infliximab (n = 101) for at least 3 months were included.

Impaired cytotoxic function and exhausted phenotype of natural killer cells in VEXAS syndrome.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disorder caused by acquired somatic UBA1 mutations in hematopoietic stem cells, affecting peripheral myeloid and natural killer (NK) cells. Given the high rate of severe infections observed in VEXAS patients, we hypothesized that NK cell dysfunction contributes to this increased susceptibility. We conducted a comprehensive immune characterization of peripheral NK cells in patients with VEXAS (n=40), patients with autoinflammatory diseases without UBA1 mutations (n=22), and elderly gender-matched healthy controls (HCs) (n=16).

Performance in adults of the EULAR/PRINTO/PRES (Ankara 2008) classification criteria for IgA vasculitis.

Objective: To examine the performance in adults of the European Alliance of Associations for Rheumatology (EULAR)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 classification criteria for IgA vasculitis (IgAV).

Methods: The EULAR/PReS/Ankara 2008 classification criteria for IgAV were applied to patients enrolled in an international observational cohort which included patients with IgAV and comparators with other forms of small-vessel and medium-vessel vasculitis. After the initial assessment of the performance of the criteria, possible revisions to increase the performance were tested.

A specific diagnostic metabolome signature in adult IgA vasculitis.

Introduction: IgA vasculitis diagnosis relies primarily on clinical features and is confirmed by pathological findings. To date, there is no reliable noninvasive diagnostic biomarker.

Objective: We aimed to explore the baseline serum metabolome of adult patients with IgA vasculitis to identify potential diagnostic biomarkers.

[Adult IgA vasculitis].

ADULT IGA VASCULITIS. IgA vasculitis previously named rheumatoid purpura is a rare systemic vasculitis in adults involving small vessels and associated with the presence of immunoglobulin A deposits. IgA vasculitis is often triggerd by infections, taking medication and vaccination.

Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients.

Introduction: This study aimed to provide an updated analysis of the different prognostic trajectories of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibodies.

Methods: Among a cohort of 70 patients, baseline characteristics and phenotypes, treatments and outcomes were analyzed. A Cox proportional hazards model was used to identify factors associated with poor outcomes, i.

No evidence for systemic low-grade inflammation in adult patients with early-treated phenylketonuria: The INGRAPH study.

The question of an increased cardiovascular risk has been recently raised in adults with phenylketonuria (PKU). As low-grade systemic inflammation increases cardiovascular risk, the INGRAPH study aimed to evaluate low-grade inflammation in adult PKU patients compared to healthy controls and to determine the potential influence of Phe-controlled diet on inflammation. Twenty early-treated adult PKU patients, including a subgroup of 15 classical PKU patients, and 20 healthy volunteers were included.

Joint manifestations revealing inborn metabolic diseases in adults: a narrative review.

Inborn metabolic diseases (IMD) are rare conditions that can be diagnosed during adulthood. Patients with IMD may have joint symptoms and the challenge is to establish an early diagnosis in order to institute appropriate treatment and prevent irreversible damage. This review describes the joint manifestations of IMD that may be encountered in adults.

New insights into epidemiological data and impact of the COVID-19 pandemic on IgA vasculitis in children and adults: a French nationwide cohort.

IgA vasculitis (IgAV) is a small size vasculitis for which epidemiologic data are strikingly lacking, especially about the adult form. Additionally, the COVID-19 pandemic seems to have profoundly modified the incidence of this disease. Here, we aimed to establish some relevant epidemiological data in both pediatric and adult IgAV.

Adverse effect of Pneumocystis Jirovecii infection associated with rituximab therapy for autoimmune disease are more frequently reported in older vs. younger patient.

Introduction: Rituximab is a chimeric anti-CD20 antibody commonly used to treat patients with autoimmune diseases. Such diseases mainly affect young people, but older patients may also be concerned. So far, very little data exist concerning the safety of rituximab in older patients with autoimmune diseases.

COVID-19 Vaccination as a Trigger of IgA Vasculitis: A Global Pharmacovigilance Study.

Objective: IgA vasculitis (IgAV) can occur after vaccination. We aimed to assess a potential safety signal on the association between coronavirus disease 2019 (COVID-19) vaccines and IgAV.

Methods: Cases of IgAV involving COVID-19 vaccines were retrieved in VigiBase.

IgA Vasculitis Following COVID-19 Vaccination: A French Multicenter Case Series Including 12 Patients.

Objective: The worldwide coronavirus disease 2019 (COVID-19) vaccination campaign triggered several autoimmune diseases. We hereby aimed to describe IgA vasculitis (IgAV) following COVID-19 vaccination.

Methods: We conducted a national, multicenter, retrospective study in France of new-onset adult IgAV diagnosis following COVID-19 vaccination.

Pleuropulmonary Manifestations of Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome.

Background: The vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a newly identified autoinflammatory disorder related to somatic UBA1 mutations. Up to 72% of patients may show lung involvement.

Research Question: What are the pleuropulmonary manifestations in VEXAS syndrome?

Study Design And Methods: One hundred fourteen patients were included in the French cohort of VEXAS syndrome between November 2020 and May 2021.

Immunoglobulin A Vasculitis Following COVID-19: A French Multicenter Case Series.

Objective: Immunoglobulin A vasculitis (IgAV) usually occurs following viral respiratory tract infection. In the context of the global coronavirus disease 2019 (COVID-19) pandemic, we describe a case series of patients who developed IgAV following SARS-CoV-2 infection.

Methods: This national multicenter retrospective study included patients with IgAV following SARS-CoV-2 infection from January 1, 2020, to January 1, 2022.

Towards better indications for kidney biopsy in adult IgA vasculitis: a clinical-laboratory and pathology correlation study.

Background: Indications for kidney biopsy in adult IgA vasculitis (IgAV) remain debated and there are very few studies on this subject. The aim of this study was to establish a correlation between renal histological and clinical-laboratory data.

Methods: A retrospective multicenter study was conducted using three databases from French hospitals, gathered between 1977 and 2020.